Recurrent epistaxis with coagulation disorders in a boy aged 2 years / 中国当代儿科杂志

A boy, aged 2 years and 5 months, had recurrent epistaxis, and the coagulation function examination showed that activated partial thromboplastin time (APTT) was significantly prolonged. Further laboratory examinations showed that the prolonged APTT was not immediately corrected in the APTT correction test, with positive lupus anticoagulant and low prothrombin activity. The boy was diagnosed with hypoprothrombinemia-lupus anticoagulant syndrome. The condition was improved after treatment with glucocorticoid, immunoglobulin, and vitamin K1. The boy has been followed up for 6 months, and no epistaxis was observed. Prothrombin activity returned to normal, and lupus anticoagulant remained positive. This is a relatively rare disease, and for patients with bleeding symptoms and coagulation disorders, it is recommended to perform the tests such as APTT correction test, lupus anticoagulant testing, and coagulation factor dilution test, which can improve the detection rate of this disease, so as to achieve early diagnosis, provide rational treatment in the early stage, and improve the prognosis.

Lupus anticoagulant: two cases report and literature review / 中华血液学杂志

<p><b>OBJECTIVE</b>To deepen the understanding of clinical manifestations and treatment of patients with positive lupus anticoagulant (LAC).</p><p><b>METHODS</b>The clinical data of 2 patients were analyzed and related literature were reviewed.</p><p><b>RESULTS</b>Case 1, a 31-year-old female, diagnosed as lupus anticoagulant positive, secondary to undifferentiated connective tissue disease, was presented with menorrhagia and thrombocytopenia. Anti-nuclear antibody (ANA) was positive 1:1000 (homogeneous type) with anti-double stranded DNA positive, and dRVVT LA1/LA2 was 3.4. Coagulation function was alleviated after treatment with glucocorticoid and total glucosides of paeony. Case 2, a 59-year-old female was presented with gingival bleeding, hematuria with the level of F II:C 13%. dRVVT LA1/LA2 was 2.0. Anti-nuclear antibody (ANA) was positive 1:1000 (type of cytoplasmic granule), anti-double stranded DNA was positive. The patient was diagnosed as hypoprothrombinemia-lupus anticoagulant syndrome (LAHS) and acquired coagulation factor deficiency. The signs of hemorrhage were alleviated after treatment with methylprednisolone 40 mg/day and cyclophosphamide, while the level of F II:C was below normal.</p><p><b>CONCLUSION</b>Symptoms of patients with positive LAC are variable. The diagnosis relies on history of disease and laboratory test. Currently, there is no standardized treatment. Cases of LAHS should be thoroughly investigated for any known causes and related disorder.</p>

Lupus Anticoagulant-hypoprothrombinemia Syndrome as an Initial Manifestation of Systemic Lupus Erythematosus: A Case Report / 임상소아혈액종양

The lupus anticoagulant-hypoprothrombinemia syndrome, characterized by presence of lupus anticoagulant with acquired factor II deficiency, is a rare disease entity often presented with acute bleeding episodes. A 15-year-old girl was hospitalized with 3 month history of menorrhagia and easy bruising. Prothrombin time (31.3 sec, normal value: 10-13 sec) and activated partial thromboplastin time (72.5 sec, normal value: 27.5-34.7 sec) were markedly prolonged and partially corrected after mixing with normal plasma. Decreased Factor II activity (4%, normal range: 79-131%) or prolonged dilute Russell's viper venom time (89.8 sec, normal value: 25.4-34.3 sec), was consistent with lupus anticoagulant-hypoprothrombinemia syndrome. Antinuclear antibody, anti-double strand-DNA antibodies and anticardiolipin antibodies were also positive. Bleeding diathesis tends to wax and wane while 5 years of treatment with steroid combined with immunosuppressants, however, there was no more active bleeding episodes. Several years after diagnosis, myocarditis, pericarditis, seizure was occurred, fulfilled the diagnostic criteria of systemic lupus erythematosus.

Lupus Anticoagulant-hypoprothrombinemia Syndrome as an Initial Manifestation of Systemic Lupus Erythematosus: A Case Report / 임상소아혈액종양

The lupus anticoagulant-hypoprothrombinemia syndrome, characterized by presence of lupus anticoagulant with acquired factor II deficiency, is a rare disease entity often presented with acute bleeding episodes. A 15-year-old girl was hospitalized with 3 month history of menorrhagia and easy bruising. Prothrombin time (31.3 sec, normal value: 10-13 sec) and activated partial thromboplastin time (72.5 sec, normal value: 27.5-34.7 sec) were markedly prolonged and partially corrected after mixing with normal plasma. Decreased Factor II activity (4%, normal range: 79-131%) or prolonged dilute Russell's viper venom time (89.8 sec, normal value: 25.4-34.3 sec), was consistent with lupus anticoagulant-hypoprothrombinemia syndrome. Antinuclear antibody, anti-double strand-DNA antibodies and anticardiolipin antibodies were also positive. Bleeding diathesis tends to wax and wane while 5 years of treatment with steroid combined with immunosuppressants, however, there was no more active bleeding episodes. Several years after diagnosis, myocarditis, pericarditis, seizure was occurred, fulfilled the diagnostic criteria of systemic lupus erythematosus.

Pulmonary hemorrhage in pediatric lupus anticoagulant hypoprothrombinemia syndrome / 소아과

Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS), a very rare disease that is caused by the presence of antifactor II antibodies, is usually counterbalanced by the prothrombotic effect of lupus anticoagulant (LAC). Patients with LAHPS are treated using fresh frozen plasma, steroids, immunosuppressive agents, and immunoglobulins for managing the disease and controlling hemorrhages. Notably, steroids are the important treatment for treating hypoprothrombinemia and controlling the bleeding. However, some patients suffer from severe, life-threatening hemorrhages, when factor II levels remain very low in spite of treatment with steroids. Here, we report a case of LAHPS in a 15-year-old girl who experienced pulmonary hemorrhage with rapid progression. She was referred to our hospital owing to easy bruising and prolonged bleeding. She was diagnosed with LAHPS that presented with pancytopenia, positive antinuclear antibody, proloned prothrombin time, activated partial thromboplastin time, positive LAC antibody, and factor II deficiency. Her treatment included massive blood transfusion, high-dose methylprednisolone, vitamin K, and immunoglobulin. However, she died due to uncontrolled pulmonary hemorrhage.

Crown removal and endodontic drainage as a last method in active gingival bleeding with liver cirrhosis and periodontitis: a case report

The most common local cause of active gingival bleeding is the vessel engorgement and erosion by severe inflammation. Abnormal gingival bleeding is also associated with the systemic disturbances. Hemorrhagic disorders in which abnormal gingival bleeding is encountered include the following: vascular abnormalities (vitamin C deficiency or allergy), platelet disorders, hypoprothrombinemia (vitamin K deficiency resulting from liver disease), and other coagulation defects (hemophilia, leukemia). There are many conventional methods for gingival bleeding control, such as, direct pressure, electrocoagulation, direct suture, drainage, application of hemostatic agents and crushing and packing. If the active continuous gingival bleeding is not stopped in spite of the application of all conventional bleeding control methods, the life of patient is threatened owing to upper airway obstruction, syncope, vomiting and hypovolemic shock. Therefore, the rapid and correct hemostatic method is very important in the emergency dental care.

Lupus anticoagulant-hypoprothrombinemia in healthy adult

The presence of lupus anticoagulant is associated with an elevated risk of venous and arterial thrombosis, and recurrent miscarriages as well. For some cases, this disease can present with bleeding as a consequence of lupus anticoagulant hypoprothrombinemia (LAHPS). LAHPS is a rare disease and it is reported to be most frequent in young females with/without systemic lupus erythematosus or in healthy children who are suffering with a viral infection. In such cases, steroid therapy is usually effective in normalizing the biological abnormalities and controlling the bleeding problems. A 34-year-old previously healthy man was admitted to our department because of his prolonged coagulation times; these abnormalities were discovered before performing orthopedic surgery. The prothrombin time (PT) was 15.2 sec, and the activated partial thromboplastin time (APTT) was 37.7 sec. A 1:1 dilution of patient plasma with normal plasma nearly corrected the PT, but this failed to correct the APTT. Evaluation of the clotting factors revealed decreased levels of factors II, V, VIII, IX and XI. The presence of LA was demonstrated by the dRVVT test, and the patient was diagnosed with LAHPS. He was successfully treated with corticosteroid before performing the orthopedic surgery.

A Case of Vitamin K1 Dermatitis due to Intralesional Injection for Cosmetic Purpose / 대한피부과학회지

Vitamin K is a lipid-soluble vitamin used in the treatment of hypoprothrombinemia found in diseases of the liver, biliary tract and small intestine. Vitamin K1 (Phytonadione) is the natural form of vitamin K. Recently, a cream containing vitamin K1 has been marketed for topical use in the treatment of periorbital hyperpigmentation, telangiectasia and rosacea. Vitamin K1 dermatitis is a cutaneous adverse reaction to vitamin K1 and can cause acute pruritic, erythematous, eczematoid, indulated plaques or slowly-appearing sclerodermatous plaques. We present a case of dermatitis caused by a vitamin K1 intralesional injection for treatment of facial telangiectasia and flushing.

Anticoagulante lúpico asociado a diátesis hemorrágica. Informe de un caso / Lupus anticoagulant (LAC) associated to hemorrhagic diathesis: case report

La paciente motivo del presente informe no cumplía los criterios para diagnóstico de LES. La presencia de ACL, serología falsamente positiva, aCL elevadas e hipocomplementemia asociada a diátesis hemorrágica, además de las respuestas clínicas y de las pruebas de la coagulación con esteroides en dosis altas, nos hace pensar que se trata de un síndrome ACL-HP. Sería además el primer caso informado con henmorragia uterina anormal.

A study of Vitamin K status in children on prolonged antibiotic therapy.

Vitamin K deficiency is known to cause coagulopathy and bleeding in patients on prolonged antibiotic therapy. This study was conducted to evaluate the status of vitamin K deficiency in hospitalized children on prolonged antibiotic therapy and its role in reversing the coagulopathy. A prospective non-randomized study was conducted on children on antibiotic therapy at a tertiary care hospital. Children in the 1 month-1 year age group developed significant coagulopathy as compared to other age groups. Coagulation abnormalities were also seen to be more in children with greater grades of malnutrition, on a more prolonged course of antibiotics and in children who were critically ill in intensive care. Hypoprothrombinemia previously reported to be due to B-lactam antibiotics containing the N-Methyl Thio Tetrazole (NMTT) group also resulted from antibiotics without this side chain. Inhibition of intestinal microorganisms by antibiotics was thought to be a likely explanation of this phenomenon. We suggest Vitamin K prophylaxis in severely ill patients, on extended periods of antibiotics and inadequate diet to prevent morbidity and mortality.

Prothrombin deficiency resulted from a homozygous Glu29 to Gly mutation in the prothrombin gene / 中华血液学杂志

<p><b>OBJECTIVE</b>To investigate the gene mutations in a pedigree with inherited prothrombin (FII) deficiency.</p><p><b>METHODS</b>The activated partial thromboplastin time (APTT), prothrombin time (PT), FII activity (FII:C) and FII antigen (FII:Ag) test were used for phenotype diagnosis. The genomic DNA was extracted from the peripheral blood of the propositus. All the 14 exons, intron/exon boundaries and the 5' and 3' untranslated regions (UTR) of the prothrombin gene were amplified by polymerase chain reaction (PCR). The PCR products were screened by direct sequencing and the mutations detected were further confirmed by restricted enzyme digestion. One hundred and three healthy blood donors were used as controls.</p><p><b>RESULTS</b>The phenotype of the propositus was prothrombin deficiency (type I). With reference to the prothrombin nucleotide sequence published by Degen & Dacie, three variations were found in the FII gene of the propositus. Among them, the novel mutation was a homozygous A601G subtitution in exon 2.</p><p><b>CONCLUSION</b>The prothrombin deficiency of the propositus is caused by a homozygous Glu29 to Gly mutation in the prothrombin gene.</p>

Insuficiencia hepática fulminante / Fulminant hepatic failure

Fulminant hepatic failure (FHF) is an acute and eventually fatal illness, caused by a severe hepatocyte damage with massive necrosis. Its hallmarks are hepatic encephalopathy and a prolonged prothrombin time (<40 percent). FHF is currently defined as hyperacute (encephalopathy appearing within 7 days of the onset of jaundice), acute (encephalopathy appearing between 8 and 28 days) or subacute (encephalopathy appearing between 5 and 12 weeks). FHF can be caused by viruses, drugs, toxins, and miscellaneous conditions such as Wilson's disease, Budd-Chiari syndrome, ischemia and others. However, a single most common etiology is still not defined. Factors that are valuable in assessing the likelihood of spontaneous recovery are age, etiology, degree of encephalopathy, prothrombin time and serum bilirubin. The management is based in the early treatment of infections, hemodynamic abnormalities, cerebral edema, and other associated conditions. Liver transplant has emerged as the most important advance in the therapy of FHF, with a survival rate that ranges between 60 and 80 percent. The use of hepatic support systems, extracorporeal liver support and auxiliary liver transplantation are innovative therapies

A Case of Systemic Lupus Erythematosus (SLE) with Antiphospholipid Antibodies Presented with Tendency for Severe Bleeding

We report a case of SLE with antiphospholipid antibodies presented initially with severe bleeding. A six-year-old boy was admitted due to severe nasal bleeding for 2 months. The boy showed typical malar rash. The laboratory tests indicated that his platelet count was 80,000/mm3 and the PT and the aPTT were markedly prolonged. A number of clotting factors were decreased, including factorsll<12%, Vll: 42%, lX : 38%, Xl: 41%, and Xll: 16%. Urinalysis showed hematuria and proteinuria, and 24-hour urine protein was 1.37g/day. Venereal Disease Research Laboratory (VDRL) test was false positive, Coombs test, lupus anticoagulants and anticardiolipin antibodies (IgG and IgM) were positive. His symptoms and laboratory tests fulfilled the criteria of SLE with antiphospholipid antibody. Renal pathology showed lupus nepritis (diffuse proliferative glomerulonephritis, class lV). After steroid therapy, his nasal bleeding stopped immediately, and laboratory findings became normalized. This case showed the tendency of paradoxic bleeding, instead of the expected thrombosis which can be found in this type of patient. We anticipate it is mainly due to pronounced prothrombin deficiency.

A Case of Systemic Lupus Erythematosus (SLE) with Antiphospholipid Antibodies Presented with Tendency for Severe Bleeding

We report a case of SLE with antiphospholipid antibodies presented initially with severe bleeding. A six-year-old boy was admitted due to severe nasal bleeding for 2 months. The boy showed typical malar rash. The laboratory tests indicated that his platelet count was 80,000/mm3 and the PT and the aPTT were markedly prolonged. A number of clotting factors were decreased, including factorsll<12%, Vll: 42%, lX : 38%, Xl: 41%, and Xll: 16%. Urinalysis showed hematuria and proteinuria, and 24-hour urine protein was 1.37g/day. Venereal Disease Research Laboratory (VDRL) test was false positive, Coombs test, lupus anticoagulants and anticardiolipin antibodies (IgG and IgM) were positive. His symptoms and laboratory tests fulfilled the criteria of SLE with antiphospholipid antibody. Renal pathology showed lupus nepritis (diffuse proliferative glomerulonephritis, class lV). After steroid therapy, his nasal bleeding stopped immediately, and laboratory findings became normalized. This case showed the tendency of paradoxic bleeding, instead of the expected thrombosis which can be found in this type of patient. We anticipate it is mainly due to pronounced prothrombin deficiency.

Baixo peso ao nascer e morbidade / Low weight at birth and morbidity

Os autores descrevem os problemas relacionados aos recém-nascidos de baixo peso, isto é aqueles que nascem com menos do que 2.500 g. Entre os problemas imediatos que acometem os recém-nascidos de baixo peso, isto é aqueles que nascem com menos do que 2.500 g, destamcam-se os seguintes: sofrimento respiratório, hipoglicemia, hipocalcemia, hipermagnesemia, defeitos congênitos e infecçÝo. O recém-nascido de baixo peso devido a crescimento intra-uterino retardado, apresenta os seguintes problemas pós-natais imediatos: síndrome da angústia respiratória idiopática (RDS), hiperbilirrubinemia, hipoglicemia, hemorragia pulmonar, maior risco a infecçSes, anomalia cromossômica e malformaçSes congênitas . O recém-nascido de baixo peso prematuro, apresenta com patologias mais importantes, a dificuldade respiratória (atelectasia, pneumonia, síndrome de membrana hialina), asfixia neonatal, enterocolite necrotizante, hemorragia cerebral, distúrbios metabílicos entre outros. O s autores chamam ainda a tençÝo, para o desenvolviento neurológico prejudicado, entre recém-nascido de baixo peso, com incidência aumentada de disfunçÝo cerebral mínima, dificuldaes na linguagem e no aprendizado

A Case of Congenital Hypoprothrombinemia

Congenital hypoprothrombinemia is a rare congenital coagulation defect. The clinical signs are manifestation of generalized tendency such as; mucosal bleeding, hypermenorrhea and post tooth extraction hemorrage. It is associated with prolongation of PT and PTT with normal thrombin time and decreased serum prothrombin level. A case with congenital hypoprothrombinemia was experienced by the authors. A 36 days old male baby was admitted with palor skin and vomiting started one day before admission. Right side brain parenchymal hemorrhage and left shift of lateral ventricle were on brain CT scan. Prologation of prothrombin time and partial prothrombin time with decreased serum prothrombin level were resulted. Serum factor I, V, VII, VIII, IX and X were within normal level. We report a case congenital hypoprothrombinemia with a brief review of relaed literatures.

Risk factors of bleeding diathesis secondary to low prothrombin complex level in infants: a preliminary report.

Twenty infants aged 2 weeks to 3 months with the diagnosis of bleeding disorder secondary to low prothrombin complex level were studied. Sixty children of the control group were matched to the cases by age +/- 2 weeks, sex and race. The ratio of boys to girls was 2.3:1. The median, mean, and range of age of the cases and controls were 43.5 days, 43.7 days, 21-73 days and 43.5 days, 46.8 days, 26-28 days respectively. Most of them were pale with a mean hematocrit of 23.55%. The partial thromboplastin time and prothrombin time were markedly prolonged. The means of vitamin K dependent coagulation factors II, VII, IX and X were 1.10%, 5.87%, 2.86%, and 4.47% of adult activity, respectively. The clinical manifestations related to the bleeding of the cases were drowsiness and convulsion (95%), pallor (85%), and apparent bleeding (10%). The sites of the bleeding were demonstrated in the cranial cavity (95%), gastrointestinal tract and oral cavity (15%), and skin (5%). Nineteen patients with intracranial hemorrhage had bleeding in the subdural space (79%), intracerebral (42%), intraventricular (32%), and subarachnoid space (5.2%). The mortality rate and permanent brain damage occurred in 10% and 45%, respectively. Only 45% of the cases recovered normally. The permanent neurological sequelaes were hemiparesis (44.4%), microcephaly (33.3%), convulsive disorder (33.3%), mental retardation (33.3%), spasticity (22.2%), and hydrocephalus (11.1%). Breast feeding alone up to the day of study (OR = 7.0, p < 0.005) was found to be a significant risk factor for bleeding in these infants.(ABSTRACT TRUNCATED AT 250 WORDS)

Dicumarol content in alcoholic herb elixirs: one of the factors at risk induced IVKD-I.

Nine medicinal plants known to be the ingredients of the traditional herbal medicinal elixir, and seven popular commercial alcoholic herb elixirs were investigated for the content of dicumarol by using high pressure liquid chromatography (HPLC) and thin layer chromatography (TLC) methods. Umbelliferae (Conioselinum Univittatum) were the only medicinal plants found to contain dicumarol 0.04 mg/dl. Dicumarol content was also found in three out of seven brands of commercial alcoholic herb elixirs with the concentration of 0.58, 1.86 and 6.00 mg/dl. These findings indicated that the traditional herbal medicinal elixirs containing dicumarol in varying amount may play a role in inducing bleeding diathesis in breast-fed infants of mothers known to consume the elixir.